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PubMed1988

Decreased expression of neuropeptides in malignant paragangliomas: an immunohistochemical study.

Paraffin-embedded sections of 99 human adrenal and extraadrenal paragangliomas were analyzed by the indirect immunoperoxidase technique for the presence of neuron-specific enolase (NSE) and 10 neuropeptides. Each showed diffuse staining for NSE. Most tumors were positive for [Leu5]-enkephalin (76 per cent), [Met5]-enkephalin (75 per cent), somatostatin (67 per cent), and pancreatic polypeptide (51 per cent), followed by vasoactive intestinal polypeptide (VIP) (43 per cent), substance P (31 per cent), ACTH (28 per cent), calcitonin (23 per cent), bombesin (15 per cent), and neurotensin (12 per cent). The neuropeptides paralleled to a large extent those normally found in the sympathetic nervous system. Clinically malignant paragangliomas (n = 25) with proven regional or distant metastases expressed considerably fewer neuropeptides, although the spectrum of those seen remained similar. Malignant paragangliomas contained an average of two neuropeptides per tumor, in contrast to five for the benign tumors (P less than 0.05). Logistic regression analysis of staining results revealed that the paucity of enkephalins, somatostatin, pancreatic polypeptide, and VIP along with the patient's sex was predictive of clinical malignancy. Our results show a definite relationship between expression of neuropeptides and the biologic behavior of these paragangliomas.

Adolescent↗

PubMed1978

Primary tumors of the external and middle ear. II. A clinicopathologic study of 14 paragangliomas and three meningiomas.

Paragangliomas (chemodectomas, glomus jugulare tumors) represented 15% of all neoplasms of the ear in the period from 1964 to 1975 at the University of Minnesota, Minneapolis. Women in the fifth to sixthe decades of life were mainly affected, and hearing loss and tinnitus were the principal symptoms. Although most tumors had the typical histopathologic appearance, two neoplasms that were initially diagnosed as paragangliomas illustrated the problems in differential diagnosis. One of these latter two tumors had metastasized and was thought to represent a malignant paraganglioma. Retrospectively, both neoplasms were adenomatous tumors of the middle ear. For the remaining patients with typical jugular paragangliomas, surgery and/or irradiation therapy controlled the local tumor in 12 cases. Two of the three meningiomas occurring in the middle ear were preceded by or associated with an intracranial component. There were only four prior examples in the literature, excluding our one case, of primary meningiomas of the ear-mastoid region. Histologically, the features were virtually identical to the usual intracranial meningioma.

Adult↗

PubMed1996

Preoperative embolization of paragangliomas and angiofibromas. Measurement of intratumoral arteriovenous shunts.

OBJECTIVE: To determine if technetium Tc 99m-labeled macroaggregated albumin selective angioscintigraphy enables estimation of microparticle-arteriovenous shunt in branchial paragangliomas and nasopharyngeal angiofibromas. DESIGN: Technetium Tc 99m-labeled macroaggregated albumin particles with calibrated diameters between 25 and 50 microns were administered during selective catheterization of the feeding arteries in 4 branchial paragangliomas (glomus tumors) and 4 nasopharyngeal angiofibromas. Preliminary measures of the activity of the tumor region and of the lungs were obtained in the angiography suite using a hand-held detector. Without changing the position of the microcatheter, definitive embolization was performed. After endovascular treatment, scintigraphy was performed and the pulmonary shunt index was calculated with the use of a gamma camera and a dedicated computer program. RESULTS: The pulmonary shunt index varied between 0% and 36% for branchial paragangliomas (glomus tumors) and between 82% and 95% for angiofibromas. CONCLUSIONS: Our results provide new evidence that the incidence of microparticle-arteriovenous shunt in nasopharyngeal angiofibromas is much higher than in paragangliomas.

Adolescent↗

PubMed2000

Paraganglioma of the urinary bladder: can biologic potential be predicted?

BACKGROUND: Paraganglioma of the urinary bladder is rarely encountered and its biologic behavior is uncertain. The authors sought to determine the prognostic factors that would predict patient outcome. METHODS: The Mayo Clinic experience over 53 years with paraganglioma of the bladder was reviewed. All histologic slides from 16 patients were reviewed by the authors. Eight cases were examined immunohistochemically with cytokeratin (AE1/3, cytokeratin 7, and cytokeratin 20), vimentin, S-100 protein, neuroendocrine markers (chromogranin, synaptophysin, and neuron specific enolase), p53 protein, and MIB-1. DNA ploidy was determined by digital image analysis in formalin fixed, paraffin embedded tissue. The mean follow-up was 6.3 years (range, 0.4-16.4 years). RESULTS: Paraganglioma usually occurred in young adult women (mean age, 45 years; range, 16-74 years). The male-to-female ratio was 1 to 3. The common symptoms and signs were hypertension and hematuria. The tumors were usually located intramurally in the lateral and posterior wall of the bladder and were multifocal in 3 cases (18%). Seven patients were treated by transurethral resection, eight by partial cystectomy, and one by radical cystectomy. T classification was T1 (1 patient), T2 (9 patients), T3 (2 patients), and T4b (4 patients). At the time of diagnosis, one patient had distant metastasis and one had regional lymph node metastasis. One patient developed metastasis 1 year after diagnosis and died of the disease 1.5 years later. None of the patients with T1 or T2 tumors had recurrence or tumor progression. All tumors were aneuploid. The mean MIB-1 labeling index was 1.5% (range, 0.03-7.0%). The tumor cells displayed immunoreactivity for S-100 protein and neuroendocrine markers and were negative for p53 (except 1 case) and cytokeratin. CONCLUSIONS: Paraganglioma of the urinary bladder occurs mostly in young adult women. Patients with tumor of advanced classification (>/=T3) are at risk of recurrence, metastasis, and dying of the disease, whereas patients in this study with T1 or T2 disease had favorable outcomes after complete tumor resection.

Adolescent↗

PubMed1997

Subglottic paraganglioma.

Paragangliomas are uncommon neuroendocrine tumors. In the head and neck region they are most commonly associated with the carotid body, vagus nerve, jugulotympanic paraganglia, and occasionally the superior and inferior laryngeal paraganglia. Laryngeal paragangliomas and subglottic paragangliomas are rare. There have been nine reported cases in the English literature of subglottic paragangliomas. We present a case of this unusual lesion and discuss histologic characteristics and surgical treatment.

Female↗

PubMed2000

Paragangliomas-- a decade of clinical experience.

BACKGROUND AND OBJECTIVES: This study is a review of 14 patients with paragangliomas between 1986 and 1996. The purpose was to determine the sites of origin, clinical manifestations and analyze the benefits of different treatment modalities. METHODS: There were 20 tumors in 14 patients. Three (21.0%) of the patients had familial history. There were 7 (50%) females and 7 (50%) males. Anatomically 14 (70%) tumors were in head and neck, 5 (25%) were in the retroperitoneum, one (5%) was in the heart. Of the head and neck tumors 9 (64.25%) were in the carotid body, 3 (21.42%) were found in the vagus, and 2(14.33%) were found in the middle ear. The tumor found in the heart was in the atrial septum. The clinical behavior of paragangliomas is determined by cellular characteristics, secreting capabilities and tumor location. The symptoms and signs depend on the site of origin and the stage at which it presents. The clinically functioning tumors were 3 (17%) in our experience and they typically present with uncontrolled hypertension. The carotid body and mediastinal tumors usually manifested as asymptomatic masses. The intravagal tumors presented with paresis of the nerve. Malignancy rarely occurs and is defined by the existence of metastasis rather than by histology. In our series 2 (10%) of the patients presented with metastasis to lymph nodes, and the vertebrae. The diagnoses in our patients were established by CT and MRI scanning. Angiography was performed in 5 patients with carotid body tumor, two of whom underwent therapeutic embolization to reduce the tumor size. The mainstay of treatment was surgical removal, though radiation has been advocated for patients who cannot undergo surgery. RESULTS: All patients underwent successful surgical resection of the tumor after appropriate preoperative preparation. Late mortality occurred in two (12.5%) patients at 3 and 5 years from unrelated etiology. Four (25%) patients were lost to follow-up. Three (18.7%) patients developed new primaries, two of them at two years and one after 8 years. One (6%) patient developed recurrent paraganglioma after remaining disease free for 20 years. CONCLUSION: In conclusion, paragangliomas are rare with multicentricity being more common in patients with familial history. The malignant potential of the tumor is determined by metastasis as there are no characteristic cellular change. Aggressive surgery is mandatory to obtain disease free survival with low morbidity and mortality. Recurrences can also be successfully operated with low morbidity.

Adolescent↗

PubMed1982

Cyanotic congenital heart disease with malignant paraganglioma.

A malignant paraganglioma of the subclavian (supra-aortic) area and organ of Zuckerkandl with metastases to the liver and pancreas was discovered at autopsy in a 22-year-old man with known transposition of the great arteries. Light microscopy showed the typical "Zellballen" pattern and Grimelius stain showed intracytoplasmic argyrophilic granules, which appeared ultrastructurally as electron dense granules. Review of the literature disclosed 59 previously reported cases of hypoxia associated with endocrine tumors. The case presented is believed to be the first example of a subclavian paraganglioma associated with hypoxemia. One previous case of a paraganglioma arising in the organ of Zuckerkandl that occurred in a hypoxic state has been reported. The possibility of cyanotic congenital heart disease with chronic long-standing hypoxia, predisposing to the development of paraganglioma with malignant transformation is presented.

Adult↗

PubMed1984

Paraganglioma of the duodenum in association with congenital neurofibromatosis. Possible relationship.

A case of a duodenal paraganglioma in a patient with von Recklinghausen's disease is presented, along with a review of the common clinical features of the 26 cases of duodenal paraganglioma and related neurogenic tumors reported in the literature. There was no reported evidence of recurrence or metastasis. Local excision was the most commonly employed method of treatment. It is proposed that duodenal paraganglioma with its admixed components and variable histologic features may represent hamartomatous hyperplasia of the paraganglia and the supporting neuroid tissue in this area. This would explain the benign behavior of these tumors as compared with carcinoids. Its association, in this case, with neurofibromatosis supports the neural crest origin of duodenal paragangliomas.

Cytoplasmic Granules↗

PubMed1986

Melanotic paraganglioma of the uterus.

Two cases of melanotic paraganglioma of the uterus are reported. Interpreted as a pigmented paraganglioma, both lesions were an incidental finding in uteri removed for unrelated benign conditions. Microscopically, both tumors were circumscribed, and composed of nests of large round or angulated polygonal cells with abundant clear or granular pale, eosinophilic cytoplasm. Large amounts of coarse intracytoplasmic melanin pigment were present in many cells, whereas other cells were completely devoid of pigment. Both lesions contained psammoma bodies. Electron microscopy showed abundant intracellular melanosomes, including many compound melanosomes and few premelanosomes. A lamellar whorled structure was the dominant infrastructure on which melanin pigment was deposited in many cells. The absence of microvilli or dendritic cell processes and basal lamina, as well as the lack of S-100 protein, pointed against a Schwannian or melanocytic differentiation. The presence of abundant pigment and only rare neuroendocrine-like secretory granules in the cytoplasm of a few cells indicated divergence from classic paraganglioma. Architecturally, this lesion is similar to a paraganglioma; but, functionally, instead of neuroendocrine granules, the tumor cells produce melanin pigment.

Adult↗

PubMed2002

National Cancer Data Base report on malignant paragangliomas of the head and neck.

BACKGROUND: Malignant paragangliomas of the head and neck are rare, with previous reports limited to nine or fewer patients. The current review included 59 cases extracted from the National Cancer Data Base that were diagnosed between 1985-1996. METHODS: The primary criterion for inclusion in the current study was verified metastatic spread from a paraganglioma of the head and neck. Patterns of presentation and treatment as well as clinically relevant associations were demonstrated in contingency tables. Relative survival was used for analysis of outcome. RESULTS: The average patient age at presentation was 44 years, and gender distribution was equivalent. Metastases were confined to regional lymph nodes in the majority of cases (68.6%), with carotid body tumors found to have an even higher rate of regional confinement (93.8%). Surgery was the most common treatment (76.3%). The use of adjuvant irradiation for regionally confined disease increased across time, from 27% (1985-1990) to 46% (1991-1996). The 5-year relative survival rate was 59.5% (76.8% for regionally confined carcinoma and 11.8% for distant metastasis). Among patients who were followed until death, those treated with adjuvant irradiation had a longer median survival (45 months) compared with those patients who were treated with surgery alone (12 months). CONCLUSIONS: Malignant paraganglioma represents metastatic spread of a tumor type that, when restricted to the site of origin, is considered benign. Metastases from malignant paragangliomas of the head and neck usually are regionally confined. The primary management of a recognized malignancy should be directed toward complete surgical removal of the primary tumor and regional lymph nodes. Postoperative irradiation may be beneficial in slowing the progression of residual disease.

Adult↗

PubMed2002

Alterations of the SDHD gene locus in midgut carcinoids, Merkel cell carcinomas, pheochromocytomas, and abdominal paragangliomas.

Several types of endocrine tumors show frequent somatic deletions of the distal part of chromosome arm 11q, where the tumor-suppressor gene SDHD (succinate-ubiquinone oxidoreductase subunit D), constitutionally mutated in paragangliomas of the head and neck, is located. In this study, we screened 18 midgut carcinoids, 7 Merkel cell carcinomas, 46 adrenal pheochromocytomas (37 sporadic and 9 familial), and 7 abdominal paragangliomas for loss of heterozygosity (LOH) and/or mutations at the SDHD gene locus. LOH was detected in 5 out of 8 (62%) informative midgut carcinoids, in 9 out of 30 (30%) sporadic pheochromocytomas, in none of the familial pheochromocytomas (0%), and in 1 out of 6 (17%) abdominal paragangliomas. No sequence variants were detected in the pheochromocytomas or paragangliomas. However, two constitutional putative missense mutations, H50R and G12S, were detected in two midgut carcinoids, which were both associated with LOH of the other allele. The same sequence variants were also detected in two Merkel cell carcinomas. In addition, the S68S polymorphism was found to coexist with the G12S sequence variant in both cases. In conclusion, we show that alterations of the SDHD gene seem to be involved in the tumorigenesis of both midgut carcinoids and Merkel cell carcinomas.

Abdominal Neoplasms↗

PubMed2006

Gross SDHB deletions in patients with paraganglioma detected by multiplex PCR: a possible hot spot?

Pheochromocytoma and paraganglioma are rare neuroendocrine tumors that arise in the adrenal medulla and the extra-adrenal paraganglia, respectively. Inheritance of these tumors is mainly a result of mutations affecting the VHL, RET, NF1, and SDH genes. Germ-line mutations of the SDH genes have been found to account for nearly 10% of apparently sporadic cases. Nevertheless, alterations other than point mutations have not yet been well characterized. In this study, we investigated the frequency of gross SDH deletions in 24 patients who tested negative for point mutations and had at least one of the recommended features for genetic testing. For this purpose, we used a technique that is easy to implement in the lab to specifically detect gross deletions affecting SDHB, SDHC, and SDHD. We identified 3 heterozygous SDHB deletions (3/24) in 3 independent cases with paraganglioma: 1 whole SDHB deletion and 2 deletions exclusively affecting exon 1. These latter mutations match the unique gross deletion previously reported, indicating this region could be a hot spot for gross SDHB deletions. It seems likely that these alterations can account for a considerable number of both familial and apparently sporadic paraganglioma cases. Although this is the first report describing the presence of gross deletions in patients with apparently sporadic paragangliomas, the extra-adrenal location of the tumor seems to constitute a determining factor for whether to include these patients in genetic testing for gross deletions in the SDHB gene.

Adolescent↗

PubMed2003

Sinonasal paraganglioma: endoscopic resection with a 4-year follow-up.

BACKGROUND: Paragangliomas are uncommon neuroendocrine tumors. In the head and neck, they are most commonly seen at the carotid body, the temporal bone, or along the vagus nerve. Sinonasal paragangliomas are extremely rare. Most cases reported describe management by open surgical resection, with sublabial and/or transfacial approaches. METHODS: We present a case of a large sinonasal paraganglioma successfully managed by endoscopic resection. RESULTS: The patient has no evidence of recurrence after 4 years of follow-up. CONCLUSIONS: This case demonstrates that sinonasal paragangliomas might be amenable to endoscopic resection. However, the most important priority to consider when selecting a surgical technique is whether adequate excision with clear margins can be obtained. Furthermore, because of the aggressive nature of these tumors, regardless of the surgical approach, long-term follow-up is recommended.

Aged↗

PubMed1989

Nasal and nasopharyngeal paraganglioma.

Paragangliomas of the nasal cavity and nasopharynx should be classified according to the presumed origin from the nasal, jugulotympanic, vagal, or ciliary paraganglion because of the substantial differences in clinical behavior and operative management. This paper presents the ninth published case of a primary nasal paraganglioma and reviews the previously reported cases. Primary nasal paragangliomas are generally localized, although the potential for local invasion is well documented. Malignancy or functional activity has not been described. Complete excision is curative. Jugulotympanic, vagal, and ciliary paragangliomas with extension into the nasopharynx or nasal cavity are typically more advanced at the time of diagnosis and require complete evaluation by computed tomography and arteriography to determine the tumor extent. The operative approach is dependant on the extent of tumor invasion. Irradiation and tumor embolization have been useful for unresectable or partially excised tumors.

Adult↗

PubMed1985

Paraganglioma of the cauda equina: a histological, ultrastructural and immunocytochemical study of two cases with a review of the literature.

The clinical and pathological features of two paragangliomas arising in the cauda equina are described and compared with 14 previous reports. The light microscopic features were similar to those of paragangliomas from other sites, with a 'Zellballen' pattern of cells containing argyrophil granules. Electron microscopy showed densely staining membrane-bound granules, cilia and fibrous bodies in the cytoplasm. The last two features only occur in paragangliomas from this site. gamma-Enolase was demonstrated by the peroxidase-antiperoxidase technique for the first time in these neoplasms. This technique was also used to demonstrate cytokeratins, which appear to be associated with the presence of fibrous bodies. The pathological findings suggest that paragangliomas in this site arise from pre-existing paraganglia, possibly of the visceral autonomic group. The prognosis in cases treated by complete excision appears to be good.

Adult↗

PubMed1978

[Biosynthesis of C19- and C21-steroids in tissue slices of paraganglioma and pheochromocytoma (author's transl)].

The biogenesis of C19- and C21-steroids has been studied in tissue slices of a paraganglioma, of a pheochromocytoma and of human adrenal cortex using radioactive steroids. Slices of paraganglioma as well as of pheochromocytoma metabolise 17-hydroxyprogesterone to cortisone, cortisol, 11-deoxycortisol and testosterone. The rate of formation of these steroids, however, by the two tumours is 8--15 times less than that in normal adrenal cortex tissue. After incubation of dehydroepiandrosterone with tissue slices of paraganglioma and pheochromocytoma, 7alpha-hydroxydehydroepiandrosterone, 11beta-hydroxy-4-androstene-3,17-dione and 4-androstene-3,17-dione were found as metabolites; testosterone was converted by both tissues to 4-androstene-3,17-dione. 17-Hydroxypregnenolone was converted to a small extent (1.7%) to dehydroepiandrosterone by slices of paranganglioma. These results show that enzymes of steroid biosynthesis (hydroxylases, oxidoreductases, delta4(-5)-isomerases, C17(-20)-desmolases) are present in both paraganglioma and pheochromocytoma.

Adrenal Cortex↗

PubMed2005

Suprasellar paraganglioma: a case report and review of the literature.

Paragangliomas arising in the suprasellar region are extremely rare. We report a case of suprasellar paraganglioma in a 47-year-old man who presented with amnesia and impaired visual acuity without any endocrine dysfunction. Magnetic resonance imaging (MRI) showed a large enhancing tumour in the suprasellar area. Following subtotal surgical excision, the diagnosis of paraganglioma was confirmed by pathology. In this case report we describe the MRI pattern of suprasellar paraganglioma and review the literature of this uncommon lesion.

Cerebral Angiography↗

PubMed2002

Loss of heterozygosity on the short arm of chromosome 1 in pheochromocytoma and abdominal paraganglioma.

Pheochromocytomas and abdominal paragangliomas are catecholamine-producing tumors that arise from sympathetic paraganglia within and outside the adrenal medulla, respectively. Deletions of the short arm of chromosome 1 have been implicated as important genetic events in their tumorigenesis and suggest a common genetic etiology. The aim of this study was to define further the chromosomal regions on 1p that are involved in the development of these tumor types. We analyzed 46 pheochromocytomas (1 benign, 6 malignant, 9 hereditary) and 7 paragangliomas (3 benign, 4 malignant) from 50 patients for loss of heterozygosity (LOH) on 1p by genotyping 15 microsatellite markers spread over the chromosome arm. Overall, LOH was detected in 33 of 46 pheochromocytomas (72%) and in 6 of 7 (86%) paragangliomas. Three minimal regions of overlapping deletions were identified: one telomeric of D1S1612(1p36.2-pter), one centromeric of D1S429 (1cen-p13), and one in the 18 cM interval defined by D1S2134 andD1S1669 (1p32). The latter region harbors the leukocyte common antigen-related (LAR) gene, which shows altered expression in sporadic rat pheochromocytomas. In conclusion, chromosome 1p may be the site of at least three putative tumor-suppressor gene loci involved in the tumorigenesis of pheochromocytomas and abdominal paragangliomas. Further studies of these regions and of LARas a candidate gene would be valuable.

Adrenal Gland Neoplasms↗